Acquired factor VIII inhibitor in a patient with mycosis fungoides.
Acquired factor VIII inhibitors have been noted in patients with hemophilia A (factor VIII deficiency), in nonhemophilic individuals with various collagen-vascular diseases, in certain normal women following parturition, and occasionally in elderly individuals with no underlying diseases. This study describes the first reported instance of a factor VIII inhibitor in a patient with mycosis fungoides who had bleeding manifested by gross hematuria. Treatment with corticosteroids and cryoprecipitate was followed by cessation of hematuria within two weeks. The patient had one episode of shoulder pain presumably related to hemarthrosis. Immunosuppressive therapy with cyclophosphamide was instituted in an attempt to decrease antibody production and control skin involvement of mycosis fungoides. Factor VIII inhibitor level rose to 100 Bethesda units without further serious bleeding. There was no peripheral blood evidence of Sézary syndrome. It is possible that some patients with cutaneous T-cell lymphomas, such as mycosis fungoides and Sézary syndrome, may have an increase in helper T-cells which may lead to excessive B-cell activity and overproduction of antibodies.
D000917:Antibody Formation; D005169:Factor VIII / Q000276:immunology*; D006395:Hemarthrosis / Q000209:etiology; D006417:Hematuria / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000097:blood* / Q000276:immunology
D005169:Factor VIII